Wednesday, July 24, 2013

If you're unfamiliar with my blog, I have a 3yo son who was diagnosed at birth with Cystic Fibrosis, a progressive and fatal genetic disease for which there is no cure. On this journey, we have the support of many family members and friends, an outstanding clinical staff, and a National Cystic Fibrosis Foundation Center that is transparent with its outcome data in an effort to promote awareness and research and fund a cure. We're getting there, but it can't happen soon enough. I spend a lot of my free time trying to think of or find ways to make life easier or do things better, things that could have an impact for my son and for other people in the CF community, and ultimately change outcomes. From medication management to insurance headaches and everything in between, I have an image of what life with this disease could look like. It would involve seamless tracking of symptoms and medications and treatments. It would include the opportunity to communicate with your doctor in between visits in a way that didn't involve phone tag. It would allow transparency in all data, allowing me to see the results of my tests and the notes that the doctors have on how I'm doing, eliminating duplication and error. I would more easily be able to find information on my disease that is relevant to me without having to stumble upon it on Facebook. And as for the things that I don't know I don't know? My doctor could be proactive in providing those things to me through the collection and organization of information that is already being shared in chat rooms and on social media. All of these things would be organized and tested and implemented so that I'm not the only person benefiting from what I want or what I want to know.

I started tracking Drew's health a couple of months ago. I record things like his weight and cough frequency as well as his appetite, stooling patterns, oxygen saturation and daily calorie intake. I've been able to share this information with his doctors to give them a more complete picture of his health in between visits, them learning about obstacles that we face in adherence to daily care regimens and me getting suggestions for ways to overcome some of those obstacles. We've worked together on additional measures to track and have discussed many ways to make life with this disease better while we wait of a cure. Being an active and engaged parent in a complicated chronic disease, what I want most is something to help alleviate the burden of care - the time it takes, the money it costs, the energy is soaks up. Enter Ginger.io.

Ginger.io is a behavioral analytics platform that turns smartphone data into health insights. I think we can all agree that people behave differently when they aren't feeling well. By collecting your behavior patterns - how often you text, the times of day you make phone calls, different movement patterns - Ginger.io aims to map behavioral patterns to create insights that, long term, have the potential to predict and prevent future problems. It's still being tested out, but I hope the Cystic Fibrosis Community and all chronic conditions will be able to benefit from a tool like Ginger.io.

Cincinnati Childrens has teamed up with Ginger.io to develop a research study for Inflammatory Bowel Disease (IBD) patients to track their symptoms. As part of this study, patients can even get paid for tracking their data. What if finding a way to treat one chronic illness like Crohns Disease, with something as simple as a mobile app, could help to find a way to treat another chronic illness like Cystic Fibrosis or Sickle Cell Disease. If you know anyone with Crohns or Ulcerative Colitis between the ages of 13-25, have them check out this study. If we all work together, everyone wins. http://ginger.io/join/c3n/

Saturday, July 20, 2013

Lots has been going on around here lately. In addition to it being a packed summer full of swim lessons and play dates and vacations, the CF Foundation has decided to move forward with a partnership with the C3N project! YEAH! We were able to share with them the value that we see if collecting and sharing new ideas and ways of managing health in CF and how the C3N project is just the place to do that. I've been keeping busy as we start to kick this thing off, creating a brand for it and figuring out a communications plan and implementations strategy. Late this week, I'm having a meeting with some folks from the foundation to share the data I collect on Drew and discuss other things that we could measure and what we could learn from the stuff we are tracking. We are also working with the UC Design School to figure out what this project needs to look and feel like so that it will work for everyone.

Drew is doing well. We had a regular clinic visit about 2 weeks ago right after we returned home from Philadelphia and our little adventure downtown for a night. His weight was down a little, which wasn't surprising given that he doesn't eat anything. We decided to start tracking his calorie intake and charting it in my PersonalExperiments with the rest of the data I collect to see what he is actually getting in and where there's room for improvement. We also started giving him Ensure Plus instead of just Ensure. I've been so focused on fat, so we decided to shift that focus to calories for a while and see what it gets us. After just two weeks of tracking, we are getting in over 2,000 calories most day, and his weight has gone up .2kg each week over the past two weeks! While keeping a food diary certainly takes some time, MyFitnessPal has made it incredibly easy for me to quickly track both what I'm giving him and then by adjusting serving size I can track what he actually takes in. The dietician can log in to see his data and his calorie distribution throughout the day, and offer suggestions based on what he seems to enjoy or what meals he seems to eat the most at. I take the total calories in a day number from MyFitnessPal and put it into the PersonalExperiments and then I can see how his stool output or cough frequency relates, if at all, to his appetite and calorie intake.

The other exciting thing that happened is that we found an electronic stethoscope company who is interested in partnering with the C3N project! They are going to let me test out the tool and we will work together to see how we can quantify breath sounds and record them and append them to the data I already collect to see what value that can bring to care. I don't have it yet but I'll definitely be updating the world once I get it and start using it.

That's all the updates I have for now. Trying to keep cool and busy this summer, and next week we head to Michigan for a little vacation. More updates as they break!

Tuesday, July 2, 2013

A few months ago, I shared a story with the CF Foundation about my vision for the future of chronic care management. If you missed it, you can read it here. Last week, I lived my "what if". Let me explain.

About two weeks ago, we packed up shop and headed east for our summer visit to the grandparents house just outside of Philadelphia. About a week into the trip, Drew developed a cough. He'd been doing pretty well and so this cough was new but not anything that I was terribly worried about. Two of his siblings had runny noses and coughs and I'd figured he had just caught the virus that they had. After a few days, the cough frequency was on the rise and his oxygen saturations were on the decline. I track many of his symptoms/behaviors with a tool called PersonalExperiments so I could see from his data that this was the case. I put in a call to his Pulmonologist back in Cincinnati suggesting we start an oral antibiotic and after some discussion she agreed that that might be the best next step.

Later that night, Drew went into some serious coughing fits like I had never heard before. He would cough and gag for hours on end, getting little to no relief from breathing treatments or airway clearance. He finally fell asleep around 2am, but woke up worn our and not much better. The next day he had another one of these intense coughing spells, and his doctor and I felt that he might benefit from an oral steroid as his symptoms seemed to indicate that there may be some serious inflammation causing the intense coughing fits. That evening, we started both the steroid and oral antibiotic and he went to bed. Then, at 11pm, 2am, 4:30am and 6:30am he was up again, coughing and gagging, still getting little relief from breathing treatments, so we decided that it was time to head to the ER.

We arrived at CHOP around 11:30am and were immediately taken back into a room. The doctors and nurses listened to my whole story and I shared with them the data I'd been collecting so they were quickly able to see what had been going on. I also gave them access to his EHR through MyChart, where they saw his bacteria sensitivities from his most recent culture and decided which antibiotics would be most effective in treating his exacerbation. We had checked in, gotten an x-ray, done a viral panel and an epiglottic culture and were admitted and moved up to a room in under 2 hours. If you've never had the opportunity to visit the ER, that quick of a conversion from arrival to admission or discharge is unheard of.

Once we were up in our room, we were greeted by a resident before we had even been screened by our nurse. She asked to see the data that I had been sharing in the ED (good news spreads quickly!). I shared my data with her and expressed my apprehensions about this being an exacerbation. An exacerbation is pretty much defined simply as a temporary worsening of the lung function due to an infection or inflammation. Although no formal definition exists, an exacerbation is generally characterized by the following symptoms:1. Shortness of breath2. Fatigue3. Increased cough4. More productive cough5. Drop in FEV1 or other markers of the pulmonary function tests

The protocol at many hospitals is to admit and treat with IV antibiotics if a patient is exhibiting these symptoms, as the assumption is made that they are suffering a pulmonary exacerbation. I did not believe Drew was having an exacerbation. While I haven't been collection data for long enough to have caught an exacerbation, I recall him being symptomatic 24/7, and Drew's serious symptoms seemed somewhat isolated to sleeping or laying down. I had notes in my tracking that called out the times that these things were happening, and at 3pm he was running around the hospital room and playing without a care in the world - asymptomatic. I thought that Drew had caught the cold that his siblings had and while he was sleeping/laying, the post nasal drip was throwing him into these coughing fits. His symptoms just didn't fit the bill of his "typical" exacerbation.

Based on the data I shared from his EHR, the attending doctor came in to discuss the IV antibiotics that they wanted to put him on. If I was going to agree to IV's, they chose the two that I would have also picked based on the positive response he had to them in the past, but I wasn't ready for IVs. Once you start two weeks of IV antibiotics, it isn't common to stop them until they're complete. I once again pulled out my data and had a discussion with the doctor about waiting to see if the steroid and/or the oral antibiotic that he is on would kick in. Since we were already in the hospital, he agreed to monitor Drew overnight before starting IV's on two conditions: I would agree to start the IV's if his oxygen dropped any lower (he was at 92) or if he had any more coughing spells overnight. He had started the steroid the night before so had only had 1 dose of both that and the oral antibiotic, usually not quite enough time for Drew to respond. He was also on inhaled Colistin as we work to eradicate his Achromobacter. The benefit of Colistin is that is covers pseudomonas and the Cipro he was on covers a number of other bacteria that he has grown in the past. It just seemed to me unlikely that he was having a true exacerbation.

So we hooked him up to a Pulse Oximeter and put him to bed. Moments later, a doctor stopped in to tell me that his culture came back positive for Rhinovirus, otherwise known as the common cold. It is known to cause a lot of inflammation and it's not unheard of for kids to have lots of coughing with this virus. Kids who have tracheomalacia, Drew, often do end up in the hospital because their airways are under attack more than they can handle on their own, and many times need a steroid to help reduce the inflammation and help them to get over the hump. For the first night in 3 nights, he slept all night long without coughing. His oxygen levels gradually started to go up on their own overnight. He woke up in the morning refreshed and looking/acting well again. I needed to take him to CHOP that morning because I was starting to reach the point of uncomfortable managing it from home in the case that things would get worse before they got better. But had I given it one more night, one more chance for the steroid to kick in and do it's job, we probably could have avoided the hospital altogether.

We were discharged at 10:30am the next morning, just under 24hrs after we had checked into the ER. Because I had been tracking his health so closely, because they were able to access his previous test results, because I felt empowered enough to speak up and express my perspective and desired course of action, and because I had given them the evidence to trust me, we found a mutually agreed upon solution that saved us two weeks in the hospital. It saved Drew the stress of being in the hospital for 2 weeks. It saved me the trouble of trying to arrange my life for a 2 week hospital stay. It saved the doctors and the hospital time and money. My insurance company wasn't being billed for unnecessary tests and an extended hospital stay.

I have data on Drew. I know the signs and symptoms of a pulmonary exacerbation. I know the difference in his cough frequency or appetite that is associated with the onset of symptoms. I know what has worked for him and what hasn't. Having that data and sharing it with anyone and everyone who might be able to better help him or benefit from it personally is why I track it. I lived my "what if" and I couldn't be more pleased with the way it turned out.

"65 Roses"is what some children with cystic fibrosis call their disease because the words are much easier for them to pronounce.

This blog, 66 Roses, is dedicated to finding the cure.

Cystic fibrosis is a life shortening genetic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). Thick mucus clogs the lungs and leads to life threatening infection. The pancreas is also obstructed by this thick mucus production, imparing digestion and leading to malnutrition. - www.cff.org